Bruton's agammaglobulinemia X linked

  • Facts:
    X linked recessive disorder
    B cell deficiency results in inability to produce Ig
  • History / PE:
    Infections begin after 6 months (when maternal ABs disappear)
    Absent tonsils
  • Diagnosis:
    B cells low/absent (low CD19+ cells)
    Normal level of T cells (CD 3+ cells)
  • Treatment:
    Regular IVIG infusion
  • Associated With:
    Recurrent lung and sinus infections with Streptococcus and Haemophilus spp.
  • Differential Diagnosis:
    Common variable immunodeficiency (acquired hypogammaglobulinemia)

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August 10th 2010